Acute Pancreatitis Secondary to Neuroendocrine Tumor

CITATION:
Hanel EG, Sovari AA, Yekkiralla L. Acute pancreatitis secondary to neuroendocrine tumor. Consultant. 2009;49(3):201.

Right upper quadrant pain of 24 hours’ duration prompted a 20-year-old man with a history of gastritis to seek medical attention. The pain was sharp and nonradiating, with no alleviating or aggravating factors. The patient occasionally consumed alcohol and regularly smoked cigarettes (tobacco and marijuana). He denied nausea, vomiting, diarrhea, and diaphoresis.

Right upper quadrant pain of 24 hours’ duration prompted a 20-year-old man with a history of gastritis to seek medical attention. The pain was sharp and nonradiating, with no alleviating or aggravating factors. The patient occasionally consumed alcohol and regularly smoked cigarettes (tobacco and marijuana). He denied nausea, vomiting, diarrhea, and diaphoresis.

A CT scan of the abdomen (Figure) revealed massive hepatomegaly with several solid lesions in the liver and a large mass in the pancreas. All abdominal organs, including the stomach, spleen, and kidneys, were compressed by the enlarged liver.

Serum tumor markers α₁ 1-fetoprotein and CA 19-9 were elevated (82.7 ng/mL and 41.4 U/mL, respectively). A CT-guided biopsy specimen from one of the liver lesions showed malignant cells suggestive of metastatic grade 2 (of 3) neuroendocrine carcinoma, consistent with pancreatic islet cell tumor. The patient was referred to a tertiary care center for further treatment. Acute pancreatitis occurs in about 3% of patients with pancreatic carcinoma.¹ Pancreatic tumors arise from either the exocrine pancreas or the endocrine pancreas; the latter is rarer (up to 2% of cases).² Neuroendocrine tumors are often found as “incidentalomas.” For the most part, imaging (increasingly with positron emission tomography) plays a key role in diagnosis and staging.³ In metastatic disease, the organs most frequently involved are the liver (70%) followed by bone and mediastinum.⁴

The most common neuroendocrine tumors of the pancreas are gastrinomas and insulinomas. Pancreatic islet cell tumors secrete a biologically active substance and result in a clinical syndrome in more than 80% of cases.⁵ In this patient, the vasoactive intestinal peptide level was 32 pg/mL; however, the gastrin level was 200 pg/mL, which might explain the previous diagnosis of gastritis. Gastrinomas account for about 20% of pancreatic islet cell tumors.⁵

Chemotherapy with streptozocin, 5-fluorouracil, and doxorubicin is used in metastatic neuroendocrine tumors. Surgical resection is indicated for focal disease. Given the age of this patient, liver transplantation with lifelong follow-up for recurrence may be an option.